Search

INTRODUCTION The risk of clinical complications in antiphospholipid syndrome (APS) increases when a patient is positive for all 3 types of antiphospholipid (aPL) antibodies. However, there is a considerable disagreement between various platforms for aCL and anti-β2-glycoprotein I (anti-β2GPI) measurement, which leads to discrepancies between these platforms in assessing aPL antibody positivity. OBJECTIVES The aim of this retrospective cross-sectional study was to assess whether 2 different platforms, the QUANTA Lite enzyme-linked immunosorbent assay and the QUANTA Flash chemiluminescent immunoassay, identify the same subjects as triple positive in a group of patients with APS and comorbid autoimmune diseases. PATIENTS AND METHODS The study included 220 patients with systemic autoimmune diseases (74 with primary APS; 47 with secondary APS; and 99 with systemic lupus erythematosus without APS). All patients were tested for IgG and IgM aCL and anti-β2GPI antibodies using both platforms. RESULTS The agreement between the positive results for individual antibodies obtained using both platforms was not full, ranging from 81.8% to 90.9% in a pair-wise comparison. However, the number of patients with triple aPL antibody positivity was similar (80 by QUANTA Lite and 86 by QUANTA Flash); the agreement between the 2 platforms for the identification of patients with triple antibody positivity was 95.5% (Cohen's kappa coefficient = 0.90). This resulted in a similar risk for APS-related clinical complications: an odds ratio of 24.9 for QUANTA Lite and of 24.7 for QUANTA Flash. CONCLUSIONS Our results confirm a strong association between triple aPL antibody positivity and APS and indicate that the identification of patients with triple antibody positivity is platform independent. When aPL antibody profiles are assessed, the agreement between various methods is much higher than that for individual antibodies.

Acquired hemophilia is a rare bleeding disorder caused by autoantibodies that inhibit coagulation factor VIII. In most cases, it manifests with severe, often life‑threatening bleeds. Acquired hemophilia may be idiopathic or secondary to another condition, most commonly other autoimmune disease or cancer. Treatment is directed to stop bleeding and eradicate inhibitory autoantibodies. Like in most life‑threatening conditions, early diagnosis and treatment are essential for good prognosis. Prompt diagnosis and treatment of acquired hemophilia are constantly improving owing to the increasing availability of laboratory diagnostic tests and growing awareness of physicians of various specialties.

Adhesive molecules, particularly selectins and integrins, are critical for the inflammatory cell trafficking from blood to the lungs. Among integrins, the most important for cell infiltration are those containing α₄ and β₂ subunits.The aim of this study was to evaluate the expression of α₁ and α₂ integrin subunits on peripheral blood T cells in asthmatic subjects, because previously we showed evidence that α₁β₁ and α₂β₁ integrins may be found on peripheral blood eosinophils in these subjects. In this study, we also analyzed the expression of α₄ and β₁ subunits as a positive reference.Expression of α₁, α₂, α₄, and β₁ subunits was analyzed by flow cytometry on CD₄+ and CD8+ T lymphocytes obtained from the peripheral blood of 54 clinically stable, asymptomatic, mild-to-moderate persistent asthmatics and 40 healthy controls.The α₁ subunit was not present on peripheral blood T cells in the majority of subjects in both study groups. Expression of α₂ was detectable on CD8+ cells in both groups and was increased on CD4+ in asthmatics. Both types of T cells showed higher expression of α₄ and β₁ in patients with asthma. Expression of α₄ was higher on CD8+ T cells both in asthmatics and controls.Expression of α₄ and β₁ integrin subunits is increased on peripheral blood T cells in patients with asthma, which confirms the preactivation of blood lymphocytes even in stable and asymptomatic disease. The biological role of α₂ subunit on T cells remains to be elucidated.

Immunoglobulin E (IgE) and the high affinity IgE receptor (FcepsilonRI) have been suggested to contribute to the pathogenesis of autoimmune disorders. Their role in Wegener's granulomatosis (WG) are, however, poorly recognized. We sought a genetic association between laboratory markers for the disease, i.e. anti-proteinase 3 antibodies (anti-PR3), anti-myeloperoxidase antibodies, anti-cyclic citrullinated peptide antibodies, C-reactive protein (CRP), C3c and C4 complement components, and total serum IgE levels in WG subjects with common genetic variants of FcepsilonRI subunits. Anti-PR3 and CRP and serum IgE levels showed significant associations, while complement components tended to be associated, with -18483A > C and/or -344C > T FCER1A (FcepsilonRI alpha-subunit gene) polymorphisms. Moreover, a correlation between -109T > C FCER1B (FcepsilonRI beta-subunit gene) genotypes and serum IgE was observed. Both WG specific auto-antibodies and other blood inflammatory markers displayed correlations with serum total IgE levels and genetic variants of the high affinity receptor for this immunoglobulin. This observation suggests a functional relantionship of FcepsilonRI in the regulation of autoimmune response observed in WG.

Autoantibodies directed against nuclear protein Ku are infrequently detected. If present, they are found in high titers in patients with connective tissue overlap syndromes. This article describes 5 patients with anti-Ku antibodies in whom systemic lupus erythematosus, Sjögren's syndrome, idiopathic lung fibrosis or scleroderma - polymyositis overlap syndrome were diagnosed. Interestingly, signs and symptoms of transient cranial neuropathy involving trigeminal and facial nerves were reported by 3 patients. Cranial nerve neuropathy has not been described in patients with anti-Ku autoantibodies previously.

Actinomycosis is a rare infectious inflammatory disease caused by the bacteria of the Actinomyces species. Infection occurs following damage to the skin or mucous membrane. This report presents a case of a 50-year-old female patient with subfebrile temperature, weight loss and pain, who used to have an intrauterine device. Masses suggestive of an advanced cancer were detected in her pelvis and abdominal cavity. A diagnosis of actinomycosis was made after histopathological examination of the tissue sample.

Venous thromboembolism (VTE) is the most common preventable cause of in-hospital death. However, the risk of VTE and prophylaxis practices in Polish hospitals are not known.The ENDORSE study in Poland was part of the global cross-sectional Epidemiologic International Day for the Evaluation of Patients at Risk for Venous Thromboembolism in the Acute Hospital Care Setting survey with the objective to assess the prevalence of VTE risk in acutely ill medical and surgical patients and to determine the proportion of at-risk patients who receive recommended prophylaxis.In 10 non-academic Polish hospitals, a chart review was performed in all inpatients aged 40 or older admitted to medical wards, and in patients at the age ofor = 18 admitted to surgical wards. The VTE risk and recommended prophylaxis were assessed according to the 2004 American College of Chest Physicians (ACCP) guidelines.The study enrolled 2673 patients (1092 in surgical wards, 1581 in medical wards). Out of these, 1111 were judged to be at risk for VTE (597 surgical patients, 514 medical patients). Only 51.8% of all at-risk patients received ACCP-recommended VTE prophylaxis (54.7% of surgical patients, 32.5% of medical patients).In Polish hospitals more than 40% of patients hospitalized for acute illness are at risk of VTE, but only a small proportion of them receives appropriate prophylaxis. These results call for decisive actions to ensure that at-risk patients receive recommended VTE prophylaxis.

The term "antiphospholipid antibodies" describing a laboratory hallmark of antiphospholipid syndrome (APS) is a misnomer. It suggests that antibodies are directed against phospholipids. Meanwhile the specific antigens are in fact proteins demonstrating an affinity to phospholipids. In APS antibodies directed against a serum protein, beta2 glycoprotein-I, play most probably a pathogenic role and are strongly associated with clinical symptoms of the syndrome. Putative mechanisms of action of anti-beta2 glycoprotein-I antibodies are discussed.

The aim of this study was to assess clinical efficacy and changes in immunological parameters during maintenance therapy with mycophenolate mofetil (MMF) in patients with lupus nephritis.Patients (n = 7) with systemic lupus erythematosus (SLE) and proliferative nephropathy confirmed by renal biopsy, in whom disease remission was induced with intravenous cyclophosphamide, received MMF (2 x 500 mg daily) for 3 months. Clinical and immunological parameters as well as drug tolerance were assessed before, and after 1 and 3 months of MMF therapy.MMF therapy lead to a decrease in disease activity index (SLEDAI), and maintenance of previously achieved reduction of proteinuria, improvement of renal function and immunological parameters. These changes were associated with decrease in the number of activated T cells and increase in B cells. The titer of anti-dsDNA antibodies was reduced in 5 out of 7 patients.Mycofenolate mofetil is effective in the maintenance therapy oflupus nephritis. A 3-month MMF treatment allows for sustained beneficial immunological profile achieved in induction therapy.

We present outcomes of 54 months' agalsidase beta enzyme replacement therapy of two 48- and 46-year-old brothers with Fabry disease. The diagnosis was confirmed in 1997, and at that moment serious damage of internal organs was observed. During enzyme replacement therapy in both brothers the following changes were observed: amelioration of gastrointestinal symptoms, gain of body weight and reduction of cardiac hypertrophy in ECG. In older brother we observed: improvement in coronary blood flow, absence of angina pectoris, dyspnea, partial remission of headache and acroparaesthesia. Reduction of proteinuria, stabilization of creatinine clearance and appearance of perspiration were also noticed. In a younger brother, with a milder form of disease, we observed: improvement of hearing and vertigo, headache reduction, as well as stabilization of kidney function (although proteinuria was slightly increased). Unfortunately, acroparaesthesia and muscle pains in the legs became more severe. Enzyme replacement therapy in Fabry disease, even started in late adulthood, is effective by stabilizing organ function and markedly improving of quality of life.

INTRODUCTION Hyperhomocysteinemia is known to predispose to atherosclerosis and occurs more commonly in patients with systemic lupus erythematosus (SLE) than in the general population. It has been shown that elevated plasma total homocysteine (tHcy) results in protein N-homocysteinylation and production o autoantibodies against N-homocysteinylated (N-Hcy) proteins. OBJECTIVES The aim of the study was to investigate whether anti-N-Hcy-albumin antibodies occur in patients with SLE and identify factors that determine these antibodies in such population. Patients and methods. In 50 subjects with SLE and 50 age- and sex-matched healthy controls, we determined serum IgG antibodies to N-Hcy-albumin using an in-house enzyme linked immunosorbent assay. RESULTS Patients had higher plasma tHcy and C-reactive protein (CRP) than controls, while serum folate and witamin B12 were lower in patients. Levels of anti-N-Hcy-albumin were higher in patients with SLE than in controls (medians: 0.31; vs. 0.19; p < 0.0001). In SLE patients, levels of anti- N-Hcy-albumin antibodies correlated with tHcy (r = 0.83; p

Classification criteria for antiphospholipid syndrome were first proposed in 1987, revised in 1999 (Sapporo criteria) and up-dated in 2006. The aim of the study was to analyze associations between clinical and laboratory symptoms of antiphospholipid syndrome in the group of patients with autoimmune diseases, based on recently up-dated classification criteria. 336 patients were enrolled into the study, with the majority (n=235) suffering from SLE. Laboratory determinations included: lupus anticoagulant (LA), anticardiolipin (aCL) and anti-beta2-glycoprotein I (abeta2GPI) (both of IgG and IgM class). Clinical and laboratory symptoms of antiphospholipid syndrome were quite common among patients studied. There was a significant association between laboratory and clinical features of antiphospholipid syndrome, according to recently modified classification criteria.

We report here the course and outcomes of 18-month enzyme replacement therapy in two 43 and 41-year-old brothers with Fabry disease. At 18 months of recombinant alpha-galactosidase A (Fabrazyme) infusions, we observed in the older patient: weight gain, decreased proteinuria (from 4 to 1.5 g/d), stabilization of creatinine clearance, much lower frequency and intensity of angina, and in the younger brother: weight gain, stabilization of creatinine clearance and proteinuria, prolongation of PQ interval and improvement of hearing. However, neurologic manifestations deteriorated over treatment period in both patients. No serious infusion-related side effects were observed.

Patients with a suspicion of non-Hodgkin lymphoma (NHL) represent a diagnostic challenge for clinicians, because they may present with a variety of symptoms. We present a series of 5 patients in whom persistent fever was one of the earliest symptoms of NHL.

We report the case of a 22-year-old male survivor of myocardial infarction as the first symptom of systemic lupus erythematosus and antiphospholipid syndrome. The diagnosis of myocardial infarction was based on typical ECG and enzymatic chanches and subsequently confirmed by technetium 99 scintigraphy. However, coronary arteries appeared to be normal at angiography. The authors conclude that myocardial infarction in SLE could be caused by a combination of cardiac microvascular thrombosis and inflammation accompanying the antiphospholipid syndrome.

We describe a case of a 52-year old woman with clinical and immunological features of sicca syndrome associated with pulmonary fibrosis, anti-Jo-1, and anti-Ro-52 antibodies but without any signs of idiopathic inflammatory myopathy.